Extensive short-bowel syndrome in neonates: outcome in the 1980s.

Fourteen infants with extensive short-bowel syndrome (SBS) were treated from 1978 through 1987 at the Children's Hospital, Columbus, Ohio. Extensive SBS was defined as a residual jejunoileum that was 25% or less than the normal expected length for each infant's gestational age. The cause of the SBS included gastroschisis (5), jejunal atresia (5), necrotizing enterocolitis (2), midgut volvulus (1), and congenital SBS (1). Infants had a mean residual jejunoileal length of 32 cm, which represented an average of 16% of normal expected jejunoileal length for gestational age. The most significant complications were associated with total parenteral nutrition (TPN): catheter sepsis (13/14 infants); cholestasis (8/14); central venous thrombosis (4/14); and cholelithiasis (3/14). The survival rate was 86%; two infants died of end-stage liver disease. Eight of 12 survivors (67%) have had intestinal adaptation develop and have discontinued TPN; three are maintained with combined TPN-enteral feeding; and one receives only TPN. Mean cost of the initial hospitalization was $315,000 with an average stay of 450 days. Survival and eventual enteral alimentation may be expected for most neonates with extensive SBS despite significant morbidity.